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Ask The Expert

January 8, 2014

Skin Infection

Question

When should a child with repeated skin infection of moderate to severe atopic dermatitis (4 to 6 courses of antibiotics annually), but with no infections elsewhere, be investigated for immunodeficiency?

Answer

By Dr. Juan Aldave Becerra:

Severe atopic dermatitis has several genetic and environmental pathogenic factors that affect the normal function of the immune system within the skin. I summarize them in the following list:

In summary, severe atopic dermatitis per se generates a state of immunodeficiency in the skin, increasing the risk for cutaneous infections in affected patients, especially by S aureus and certain herpes virus (herpes simplex virus, varicella zoster virus). Additionally, there are gene defects and variants associated with severe atopic dermatitis, such as desmoglein 1, MATT1, filaggrin, TLR2, TLR4 and SPINK5.

On the other hand, we should note that some primary immunodeficiencies (PI) such as the hyper-IgE syndrome (HIES), Wiskott-Aldrich syndrome (WAS), and Netherton syndrome may initially present as severe atopic dermatitis, resulting in delayed diagnosis and treatment. There are no established clinical criteria that can absolutely exclude PI in patients with AD. However, in every patient with severe AD we should investigate family history of PI, positive history of recurrent infections apart from the skin, and other clinical details (e.g. coarse facial features, pneumatoceles, skeletal abnormalities, delayed shedding of primary teeth, low TH17 numbers and NIH score ≥40 in STAT3-defective HIES; thrombocytopenia with microplatelets in WAS; ‘bamboo hair’ in Netherton syndrome). Even more, we must consider that the clinical manifestations of PI are highly variable depending on the type of gene mutation, the degree of protein affection, gene-gene interactions, and the effect of environmental factors.

Please look below at a list of interesting articles that will help you.

References:

Juan Aldave Becerra, MD
Hospital Nacional Edgardo Rebagliati Martins
Lima, Peru


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